Patients with the Nevus of Ota have increased amounts of melanin (pigment) and melanin producing cells (melanocytes) in and around their eyes. This includes the intraocular blood vessel layer called the uvea (choroid, ciliary body, and iris), on the white part of the eye ball (sclera), and in the eyelids.
Patients with the Nevus of Ota have been shown to be at greater risk for the development of intraocular and central nervous system malignant melanomas (e.g. choroidal melanoma). Though intraocular melanomas are more common in these patients, their lifetime risk is though to be 4%.
Patients with the Nevus of Ota should be examined at least every 6 months by an eye-cancer specialist and a neurologist.
The Nevus of Ota appears as increased pigmentation of one eye and its eyelids. The eyelid is darkened, pigment can be observed on the white of the eye and that iris may be darker (compared to the contralateral iris). Though none of these findings produces physical symptoms, physical asymmetry can have a psychological impact on the patient.
Ocular Melanosis is diagnosed in cases of ocular melanosis there is no eyelid involvement.
Careful inspection of any patient with two different colored irises (heterochromia irides) may uncover the eyelid and episcleral pigmentation diagnostic of the Nevus of Ota.
Slit-lamp biomicroscopy is used to distinguish between conjunctival and episcleral pigmentation. Comparative examination of the irides can be used to evaluate coloration and surface characteristics.
Dilated ophthalmoscopy similarly demonstrates asymmetric “darker” pigmentation of the choroidal vascular layer beneath one retina.
Ophthalmic ultrasound imaging will reveal thickening of the vascular uveal layer of the eye. Examinations should include both posterior and anterior segment (UBM) imaging.
Most patients with the Nevus of Ota have no subsequent related problems.
Less than 4% can develop choroidal melanoma in the affected eye. Fewer will develop intracranial neoplasia. Therefore, it is reasonable for all patients with the Nevus of Ota to have twice yearly eye examinations with dilated ophthalmoscopy. They should also have an initial neurology assessment, followed by periodic medical evaluations.
Choroidal osteoma are boney tumors that can grow within the eye. They arise within the blood-vessel layer “choroid” beneath the retina. Choroidal osteoma are usually found near the optic nerve and can cause vision loss. The choroidal osteoma impedes the circulation to (and harms) the overlying retina and can be associated with overlying retinal atrophy and adjacent subretinal neovascularization (or, the formation of new blood vessels).
Most choroidal osteomas do not cause any symptoms. They are found on routine eye examination. However, they can be associated with the development of subretinal neovascularization (particularly at the edges of the osteoma). If in the center of the macula, the blood and neovascularization can cause distortion or loss of vision.
A choroidal osteoma is characterized by bone in the choroidal vascular layer of the eye. These bone deposits are more likely to be found near to the optic nerve, and they are relatively flat (typically less than 2 mm thick). By looking into the eye (i.e., performing an ophthalmoscopy), your doctor will see the yellow tumors, scalloped edges, formed vessels within the tumor, some pigment clumping on its surface, and, rarely, blood associated adjacent subretinal neovascularization.
In the adult or peripheral form of the disease, the doctor might just see the tumor eroding through the overlying retinal pigment epithelium like the tip of an iceberg. In these cases, the extent of the base of the tumor might only be measured by ultrasound imaging.
Photography is used to document the choroidal osteoma size and shape. Baseline photographs are helpful for subsequent evaluations for growth and neovascularization.
A few tests additional tests may be performed in order to confirm diagnosis:
Fluorescein angiography: Since a choroidal osteoma is made of bone, when fluorescein dye circulates through the eye, it will be slow to penetrate the tumor. Once the fluorescein dye is inside the choroidal osteoma, it also has trouble getting out. This is why the fluorescein pattern of choroidal osteoma is one of slow uptake and persistent “late” fluorescence.
Ultrasound: Ultrasound examination of an eye with a choroidal osteoma will demonstrate intense reflectivity from the hard bony tumor with resultant shadowing of the posterior orbital contents. If the intensity (gain) of the ultrasound machine is decreased, the eye-care specialist is likely to see persistentm characteristic very high reflectivity or “brightness” from the tumor. Ultrasound can also be used to measure the thickness of a choroidal osteoma.
Radiographic imaging: Computed tomography (CT) can be particularly helpful to see the calcification of a choroidal osteoma in unusual cases.
Choroidal osteoma is a benign tumor; therefore, most patients do not require treatment. Choroidal osteoma related vision loss is not usually treatable. On occasion, a choroidal osteoma may be associated with subretinal neovascularization (abnormal blood vessels beneath the retina), which are typically treated with laser.When these abnormal vessels are on top of the tumor or near the central vision, intraocular anti-VEGF drugs may be advised.
Eye tumor specialists request that choroidal osteoma is monitored with periodic dilated ophthalmoscopic examinations.
Like a raised freckle on the skin, nevi can also occur inside your eye. The most common “choroidal nevus” or eye nevus are unusual and can only be seen by an eye care specialist. Like a nevus on the skin, a choroidal nevus can grow into a malignant melanoma.
A choroidal nevus rarely requires treatment. Photography is typically used to document the size of the choroidal nevus. If the choroidal nevus has orange pigmentation, if the nevus is leaking fluid, or has a thickness of 2 mm or more it may be (or become) a malignant choroidal melanoma.
Depending on its appearance, patients with a choroidal nevus should have their eyes examined (at least) every 6 months. Only your doctor can look inside your eye to see if the choroidal nevus has changed. If the choroidal nevus has orange pigment or has thickened, it should be checked more often. If a choroidal nevus is leaking subretinal fluid, this is a particularly ominous sign. Such tumors should be followed most closely for evidence of growth or malignant transformation into a choroidal melanoma.
It is reasonable to have an eye cancer specialist check to see if your choroidal nevus looks suspicious and take baseline measurements. This examination may include the use of ultrasound, specialized photography, optical coherence tomography or an intraocular angiogram. It is a good idea for each patient to keep a picture of your choroidal nevus. This picture can be compared to future examinations to help determine if the nevus has changed or stayed the same.
A benign choroidal nevus (eye freckle) rarely causes symptoms. However, if a choroidal nevus leaks fluid or is associated with the growth of abnormal blood vessels (neovascularization) patients can become symptomatic. Such changes can cause a localized retinal detachment/degeneration, flashing lights and loss of vision.
A typical choroidal nevus is asymptomatic or “causes no symptoms” and found by routine dilated eye examination with ophthalmoscopy.
Choroidal nevus is typically a pigmented tumor of the blood vessel layer (choroid) beneath the retina. A choroidal nevus is typically gray but can be brown, yellow or variably pigmented. Your eye care professional will look to see if the choroidal nevus is raised (has thickness), orange pigment (lipofuscin), or is leaking fluid (retinal detachment). If the choroidal nevus has one or more of these findings, it is labeled a suspicious choroidal nevus that has a chance of turning into or even being a small choroidal melanoma.
A choroidal nevus can have yellow-white spots on its surface called drusen or drusenoid retinal pigment epithelial detachments (DRPED).These are signs of retinal dysfunction. The nevus may be preventing the eye from removing retinal waste products or creating microscopic leaks beneath the retina.There are no studies that show how long it takes for drusen to form on a choroidal nevus.
A benign choroidal nevus requires no treatment and there is no way to safely remove them. Since a choroidal nevus can turn into a choroidal melanoma, it is reasonable to have it periodically observed by your eye care professional.
Dr. Finger believes that since skin and conjunctival melanomas have been linked to ultraviolet exposure, and since choroidal melanomas are more commonly found in patients with blue eyes, outdoor occupations, and in Australia (where there is an ozone hole); it is reasonable to wear ultraviolet (UV) blocking sunglasses. Dr. Finger says, “Think of sunglasses as sun block for your eyes.”™
Malignant tumors from other parts of the body can spread into and around the eye. Though they are the most common intraocular cancer in adults, they usually go undetected. This is because most ocular metastases go undetected unless they affect vision, are visible to the patient, or push the eye forward. Eye metastasis are most commonly found in the vascular choroid (choroidal metastasis) within the eye.
Ocular or eye metastasis are usually from a breast cancer (in women) and lung cancer (in men). Other less common sites of origin include the prostate, the kidney, the thyroid, and gastrointestinal tract. Blood cell cancer (lymphoma and leukemia) can also metastasize into the eye and orbit. In 18% of patients, the primary source of the metastasis may be undetectable. In these cases, Dr. Finger may have to biopsy the ocular tumor to obtain tissue for pathology (to determine the primary source).
As stated, most patients with choroidal metastasis have no symptoms. However, if the metastasis is on the eye or eyelids, it may be visible. If the metastasis is located behind the eye (in the orbit), the eyeball may be visibly displaced out or to the side. If the metastasis is within the eye (the most common), metastasis patients can can have symptoms of flashing lights, floating spots or distortion of their vision. In that waiting until patients are symptomatic may be too late to preserve vision, Dr. Finger suggests that all patients with a history of metastatic cancer should have periodic eye examinations.
In that most patients with ocular metastasis have either a known primary cancer and/or metastatic tumors in other parts of their body; a careful medical history is important. Patients know of prior cancer history and many can remember their tumor stage. History taking can also uncover the signs or symptoms of these other cancers. If an eye cancer specialist suspects ocular metastasis, both eyes and orbits should be examined because ocular metastases can be both bilateral and/or multifocal.
Choroidal metastasis is usually non-pigmented (except metastatic melanomas), and has typical ultrasound and angiographic patterns. Choroidal metastasis is usually poorly circumscribed and can cause retinal detachments. They may have spicules of pigment on their surface. Fluorescein angiography may reveal a typical, “starry sky” of hyper-fluorescent micro-aneurysms. Unlike primary choroidal melanoma, they can grow quickly (weeks) and may require prompt treatment.
The patient with metastasis to the eye should also be examined by a medical oncologist. A complete metastatic survey should be performed to “stage” the patient (to see if there are other tumors within the body). Specifically, radiographic imaging including but not necessarily limited to the brain and lung should be performed due to a high concurrent incidence of intracranial and pulmonary metastases. Dr. Finger will likely suggest a total body PET/CT with fusion.
Most patients with ocular metastasis have either a known primary cancer and/or metastatic tumors in other parts of their body. A careful medical history can uncover the signs or symptoms of these other cancers. If an eye cancer specialist suspects ocular metastasis, both eyes and orbits should be examined because ocular metastases are commonly both bilateral and/or multifocal.a
Choroidal metastasis are usually non-pigmented, yellow or white (except metastatic melanomas). Choroidal metastasis is usually poorly circumscribed and can cause retinal detachments. They may have spicules of pigment on their surface. Unlike primary choroidal melanoma, they can grow quickly (weeks) and may require prompt treatment. They typically have typical ultrasound findings of variable reflectivity, angiographic (starry sky microaneurysm) and OCT wavy surface patterns that are helpful to establish the diagnosis.
Treatment depends on the type of metastasis and its primary source. Treatment options often include chemotherapy, immunotherapy and radiation therapy. In general, chemotherapy and immunotherapy take a relatively long time to work, whereas radiation therapy is more rapid. The choice of chemotherapy, immunotherapy or radiation therapy typically depends on the type of tumor and the temporal “timing” risk for loss of vision.
Q: If I have been diagnosed with cancer, and have no symptoms should I get my eye checked? A: It is reasonable to have a yearly-dilated eye examination (with examination of your retina).
Q: If I am on chemotherapy do I need to have radiation too? A: If chemotherapy is controlling your eye tumor you may not need additional radiation therapy.
Q: If I have a metastatic cancer in one eye, can another occur in my other eye? A: Yes, you should be monitored every 3 to 4 months.
Tumors of the eyelids may be benign cysts, inflammations (styes), or malignant tumors (skin cancers). The most common type of eyelid cancer is basal cell carcinoma. Most basal cell carcinomas can be removed with surgery. If left untreated, these tumors can grow around the eye and into the orbit, sinuses and brain. Other eyelid cancers include squamous cell carcinoma, sebaceous cell carcinoma, and malignant melanoma. Together, these tumors make up the remaining 10% of eyelid malignancies.
Most patients with eyelid tumors will notice a nodular growth on their eyelid. This growth can be skin colored, red, brown or black. Malignant tumors may cause loss of eyelashes or distort the position of the eyelid. All patients with eyelid tumors should be evaluated by an eye care specialist with experience in the care of eyelid tumors.
A simple biopsy can determine if an eyelid tumor is malignant.
Malignant tumors are completely removed and the eyelid is repaired using plastic surgery techniques. Some doctors are now using topical immunotherapy or chemotherapy agents to avoid surgery. Dr. Finger can sometime use “Finger-tip” cryotherapy (freezing-therapy) or radiation therapy, depending on the extent of the tumor.
A Typical Work-up for a Suspected Malignant Eyelid Tumor:
The wall of the eye has 3 main layers. From outside to inside there is: the white sclera, a blood vessel layer called the uvea (choroid, ciliary body and iris) and an inner retinal layer. Further, the pigment producing cells, “melanocytes” are primarily found in the vascular uveal layer. It is those melanocytes that can turn into malignant melanoma. Therefore, when melanoma happens in the choroid, they are called “choroidal melanoma,” the most common primary intraocular malignancy in adults. That said, choroidal melanomas are rare with 5 to10 out of each million people diagnosed with a choroidal melanoma each year. Choroidal melanomas can spread to other parts of the body.
Eye cancer specialists can determine if you have a choroidal melanoma by performing a complete eye examination with testing. This includes asking questions about your medical history, examining both of your eyes, looking into the eye through a dilated pupil, performing an ultrasound examination, and specialized photography (to examine the circulation within the choroidal melanoma).
MOST – Fingers’ Melanoma Mnemonic
Dr. Finger has developed the mnemonic device “MOST” to help eye care specialists to determine if the intraocular tumor is a melanoma.
“O,” Orange Pigment Lipofuscin (OPL) a metabolic side product of cell death. This finding tells us that that either the underlying tumor is destroying the overlying tissue or itself is degenerating. Lipofuscin is best seen with a photographic test called Fundus Auto Fluorescence imaging, or FAF.
“S,” Subretinal fluid (SRF) is created by poorly formed or new, “neovascular” blood vessels within the tumor. Cancers need new vessels in order to grow. Large amounts of SRF can be seen by ophthalmoscopy (looking into the eye) and ultrasound imaging. However, small amounts of SRF are best seen on 3D optical coherence tomographic imaging (3D-OCT).
“T,” Thickness of the tumor has been associated with malignancy. Simply, the thicker it is the more likely a pigmented intraocular tumor is malignant. It is widely accepted that tumors greater than 2.0 mm are more likely to be malignant. Ultrasound imaging is currently the best method to measure tumor thickness.
Your specialist will also request that you have a complete general medical check up and specific tests depending upon what they see inside your eye. In the Collaborative Ocular Melanoma Study (COMS), participating eye cancer specialists correctly diagnosed select choroidal melanoma in over 99.6% of cases (without a biopsy). That said, patients with unusual appearing “atypical” tumors were not entered into the study.
Classic Indications for Biopsy
Atypical tumor, metastatic tumor with no observable primary cancer and when the patient requests a pathology diagnosis. More recently, primarily due to genetic testing services, more and more centers are routinely performing choroidal tumor biopsy primarily for genetic tumor analysis. Genetics offers information about the tumor, but does not allow doctors to avoid treatment or follow up systemic testing for metastasis.
Choroidal biopsy has been associated with a risk of hemorrhage, infection, retinal detachment and a poorly quantified risk of tumor seeding (outside the eye). Risks related to tumor seeding are thought to be small, but clearly they have not been evaluated by any large prospective or retrospective study. Each eye cancer specialist should discuss the relative risks (known and unknown) of biopsy prior to surgery.
Questions About Intraocular Biopsy:
Remove the need for surgical tumor treatment?
Reduce the number of radiologic examination or years needed for systemic surveillance?
What are the risks of biopsy (hemorrhage (e.g. vitreous, subretinal, subfoveal), seeding, damage to the lens, optic nerve, retinal detachment, cataract, epiretinal membrane, loss of vision, loss of eye and/or reaction to anesthesia).
Most choroidal melanoma patients have no symptoms. The melanoma is found on routine eye examination. If patients have choroidal melanoma symptoms, they are usually seeing “flashes of light,” noticing “distortion” or loss of vision, and floating objects (floaters) in their vision.
If the choroidal melanoma is in the front of the eye (near the natural lens), it can push or tilt the natural lens causing an irregular astigmatism (blurring of vision).
Choroidal melanoma can leak fluid beneath the retina, making the retina detach and cause symptoms of flashing lights and floating specks “floaters”.
If the choroidal melanoma is in the macula (center of vision), it can grow beneath the fovea making the patient far-sighted. The choroidal melanoma can also grow into and destroy the fovea causing distortion, loss of vision or changes in color perception.
It is important to note that most patients with choroidal melanoma have no symptoms at all. Their tumors are found when they visit their eye doctor for a “routine” eye examination. So everyone should have periodic eye examinations (including dilated ophthalmoscopy). In general, the earlier and smaller the choroidal melanoma, the better the prognosis for both life and sight.
Other, more unusual presentations of anterior choroidal and iridociliary melanoma are discoloration of the iris, a brown spot on the outside of the eye, an irregularly shaped pupil and glaucoma.
Choroidal melanoma is usually seen by ophthalmoscopy (when your eye doctor looks through a lens into your dilated pupil). Choroidal melanoma has typical “diagnostic” characteristics that include but are not limited to: pigmentation, low to moderate internal ultrasound reflectivity, clumps of orange pigment lipofuscin on its surface, leakage of subretinal fluid, or retinal detachment (on or around the choroidal melanoma) and thickness.
Pigmentation is due to naturally occurring melanin that comes from melanocyte cells in the choroidal layer of the eye. Choroidal melanomas are most commonly pigmented, but can be variably pigmented and even non-pigmented (amelanotic). Non-pigmented choroidal melanoma is due to a proliferation of melanocytes that have lost their ability to make the melanin pigment.
Orange pigment is made up of a chemical called lipofuscin and appears on the surface of choroidal melanomas. Lipofuscin is a product of cell death which indicate that cells are dying on the tumor’s surface. This is also sign of metabolic activity. Melanomas are more metabolically active than choroidal nevi.
Ultrasound is typically used to measure the choroidal melanoma size, evaluate internal tumor reflectivity, and look for melanoma extension behind the eye into the orbit called extrascleral extension. Ultrasound imaging has demonstrated that most choroidal melanomas are shaped like a dome and less commonly like a mushroom. Ultrasound can also evaluate and detect choroidal melanoma associated retinal detachment. However, optical coherence tomography (OCT) is a more sensitive way to detect subretinal fluid – retinal detachment.
Chaired by Dr. Paul T. Finger, a committee of top ophthalmic specialists assembled to form the AJCC-UICC Ophthalmic Oncology Task Force. To ensure a broad range of specialists, Dr. Finger “internationalized” the committee, including over 58 members both from the USA and around the world.
This committee had one driving goal: to design a clinically useful Tumor-Node-Metastasis (TNM) based classification “language” for ocular tumors. This first-of-its-kind classification system has become a universal language for all who diagnose and treat ocular tumors.
Not only does a universal classification system offer cancer staging for the patient, it also allows physicians to directly compare data. In the long run, a common “eye tumor” language helps usdetermine and differentiate treatment types as well as coordinate the efforts of researchers working for a cure.
Dr. Finger has since translated this staging system for the worlds’ Union International for Cancer Control (UICC), and offered it to the world. In order to get everyone to employ this new language, he has recruited all the major medical journals to require eye cancer researchers to use AJCC-UICC staging.
Small Choroidal Melanoma (AJCC T1 and T2): Patients with a small choroidal melanoma can be treated after their first visit, but since growth helps to prove that the tumor is a cancer, your doctor may suggest “observation” or watching for a small amount of choroidal melanoma growth prior to treatment. Your eye cancer specialist should discuss the relative risks and potential benefits of “observation for growth” as compared to “immediate treatment” for choroidal melanoma. If growth is documented (typically within 6 months of observation), eye cancer specialists will typically recommend definitive treatment.
Medium-sized Choroidal Melanoma (AJCC T3 and T4): Most patients with large-sized choroidal melanoma can be also be treated with eye-sparing low energy radiation therapy (e.g. palladium-103). However, larger tumors require more radiation and larger irradiated intraocular volumes resulting in greater risk of radiation side-effects and poor vision. Rarely such eyes have to be secondarily removed. Eye cancer specialists try to preserve eyes, even if the eye had reduced vision.
Large-sized Choroidal Melanoma: Very large choroidal melanomas (greater than 22 mm width) may be treated by initial removal of the eye (enucleation). This is because the amount of radiation required to destroy a choroidal melanoma that fills most of the eye will likely be too much for the eye to tolerate.
However, most patients, even with very large-sized choroidal melanoma can be treated with eye-sparing radiation therapy. However, after eye sparing radiation for very large choroidal melanomas, eyes are at greater risk to have poor vision, secondary inflammation and may require secondary removal at a later date.
Patients often ask why they have a choroidal melanoma. While there is no one cause, choroidal melanoma is more common among patients with blue vs. brown eyes, those with outdoor occupations, and in Australia where there is a hole in the ozone layer. Therefore, though this hypothesis has yet to be proven, it seems reasonable to assume that choroidal melanoma is related to sunlight (ultraviolet exposure).
In that sunlight exposure has been linked to several eye cancers and diseases of the eye, Dr. Finger suggests that you “Think of Sunglasses as Sun Block for your Eyes” ™ and start wearing your UV blocking sunglasses. They make great gifts too!
Dr. Finger also often gets questions related to stage and spread. These two things are closely linked––choroidal melanoma size is most closely related to its risk for spread to other parts of the body (metastasis). In three separate studies, cumulatively involving almost 20,000 patients, the average rate of metastasis has been 50%. However, patients with smaller tumors have much lower rates compared to larger tumors. Therefore, patients should ask their eye cancer specialist about their tumors AJCC-UICC tumor size and risk for metastasis. In general, the larger the choroidal melanoma the worse the prognosis for both vision and metastasis.
Hemangiomas can grow within the blood vessel layer beneath the retina called the choroid. If they are located in the macula (center of vision) or they leak fluid (which causes a retinal detachment or cystic changes in the retina), they can affect visual acuity. However, many choroidal hemangiomas never grow or leak fluid and can be observed without treatment. Choroidal hemangiomas are not cancers and never metastasize.
On your left, there is a red colored choroidal hemangioma just above the round optic nerve. Typically, choroidal hemangioma are reddish to orange colored. They can have areas of increased pigmentation which can make them difficult to differentiate from choroidal melanomas.
By lifting the overlying retina, choroidal hemangiomas can cause far-sightedness (hyperopia), distorted vision (metamorphopsia), flashing lights, or blurred vision. Choroidal hemangioma that cause no symptoms at all, are usually found on routine dilated eye examinations (ophthalmoscopy).
Intraocular Photography and Angiography: An eye care specialist can perform studies of the blood flow within the eye by injecting drugs called fluorescein or indocyanine green. They can also look at the overlying blood vessels with computerized optical coherence tomography – angiograpy (OCT-A). When drugs are injected into the arm or hand, they will travel into the blood vessels inside the eye. Using special lights that make the angiographic dyes fluoresce, your doctor can see special differentiating circulation patterns, that help differentiate between the types of tumors. For example,choroidal hemangioma have a unique pattern of circulation where the large blood vessels produce a “COARSE VASCULAR PATTERN.”
Ultrasound Imaging: Sound waves can also be used to examine the inside of the eye. Ultrasound images of a choroidal hemangioma reveals that they are “bright.”This is because they are a mass of relatively large and well-formed blood vessels. Each blood vessel reflects sound waves producing characteristically intense reflections or moderately “MODERATELY HIGH INTERNAL REFLECTIVITY” from within the hemangioma tumor. Moderately high internal reflectivity is an important diagnostic characteristic.
When a choroidal hemangioma is not causing symptoms, leaking or damaging the eye; it can be photographed and followed for evidence of change prior to treatment. However, if the choroidal hemangioma is documented to grow into the center of vision or is causing a retinal detachment or angle-closure glaucoma it is treated to save vision.
Hot Laser: Laser photocoagulation had been used to decrease the amount of fluid leaking out of choroidal hemangioma. Unfortunately, laser results are usually temporary and recurrence of retinal detachment or cystoid retinal degeneration usually results vision loss.
Photodynamic Therapy (PDT) is a cold or non-thermal laser treatment that involves injecting a light-sensitive dye into the patient and then shining a dye-activating PDT laser light on the choroidal hemangioma. The light-activated dye causes the abnormal blood vessels to close, shrink, and stop leaking. However, PDT requires that the tumor can be visualized during treatment. So, PDT will not work if there is a large retinal detachment covering the tumor or if the tumor is located too far anterior inside the eye. When it works, PDT offers a more long-term method to treat leaking posterior choroidal hemangiomas. The dye is expensive and multiple treatments are usually required. Lastly, due to changes in treatment patterns for AMD, fewer and fewer PDT laser machines and experienced PDT-laser surgeons exist.
Radiation Therapy: At The New York Eye Cancer Center most patients are treated with low-dose external beam or implant radiation therapy. Radiation has been used used to treat leaking choroidal hemangiomas for decades. The differences are, that in the past relatively large doses of radiation were used and some collateral radiation damage occurred. However, modern radiation techniques involve much lower doses of radiation as well as implant techniques that better focus the treatment area. Low dose radiation typically requires a total of less than two weeks of daily treatments. In Dr. Fingers’ experience, almost all choroidal hemangiomas and their associated retinal detachments can be cured with low dose radiation therapy.
Choroidal Hemangioma are benign tumors made up of blood vessels. Since choroidal hemangioma do not metastasize (spread to other parts of the body), we are more concerned with damage they can cause within the eye. Choroidal hemangioma can be stable and not require treatment. Others can change a patient’s vision by displacing the retina causing hyperopia (far-sightedness), causing degeneration of the overlying retina (cystoid degeneration), or by leaking fluid under and detaching the retina (serous retinal detachment).
Most choroidal hemangioma are treated if they induce a retinal detachment. Also in rare cases, choroidal hemangioma can fill most of the eye, affect the iris or be associated with the Sturge-Weber Syndrome. In these cases, the choroidal hemangioma (due to its increasing thickness) can cause angle closure glaucoma. In these cases, radiation can be used to cause regression and thinning of the hemangioma in the iris and prevent vision loss.
Malignant tumors from other parts of the body can spread in and around the eye. These tumors may never be discovered unless they affect vision, are visible to the patient, or push the eye forward. The most common location for ocular metastasis, in the vascular layer called the choroid (choroidal metastasis) within the eye.
Cancer metastasis that appear in and around the eye are usually from a breast cancer (in women) and lung cancer (in men). Other less common sites of origin include the prostate, the kidney, the thyroid, and gastrointestinal tract. Blood cell cancer (lymphoma and leukemia) can also metastasize to the eye and orbit. Once a patient is diagnosed with choroidal metastasis, we try to find where it came from. In 18% of patients, we do not find the source of choroidal metastasis. In these cases, we may have to biopsy the ocular tumor and look at its cell-type.
Most patients with choroidal metastasis have no symptoms. If the metastasis is on the eye or eyelids, it may be visible. If located behind the eye (in the orbit), the metastasis can push the eyeball out or to the side. If within the eye (the most common), choroidal metastasis patients can see flashing lights, floating spots or distortion of their vision. Patients with a history of cancer are at greatest risk and should have periodic eye examinations.
Most patients with metastasis have either a known primary cancer and/or metastatic tumors in other parts of their body. A careful medical history can uncover the signs or symptoms of these other cancers. If an eye cancer specialist suspects ocular metastasis, both eyes and orbits should be examined because ocular metastases can be both bilateral and/or multifocal.
Choroidal metastasis is usually non-pigmented (except metastatic melanomas), and has typical ultrasound and angiographic patterns. Choroidal metastasis is usually poorly circumscribed and can cause retinal detachments. They may have spicules of pigment on their surface. Unlike primary choroidal melanoma, they can grow quickly (weeks) and may require prompt treatment.
The patient with metastasis to the eye should also be examined by a medical oncologist. A complete metastatic survey should be performed to “stage” the patient (to see if there are other tumors within the body). Specifically, computed radiographic imaging of the brain and lung should be performed due to a high concurrent incidence of intracranial and pulmonary metastases. Your doctor may suggest a total body PET/CT with fusion.
The care of patients with metastasis to the eye typically involves cooperation between the eye cancer specialist, medical oncologist, and radiation therapist. Though chemotherapy can be used in many cases of orbital and choroidal metastasis, radiation therapy is usually a more definitive treatment. If the metastatic tumor has not destroyed the center of the retina, early treatment offers the best hope for preserving vision. Almost all patients with choroidal metastasis can be treated with external beam radiation. That is, surgery is rarely needed as treatment for choroidal metastasis. Orbital and adnexal ocular metastasis are typically biopsied prior to treatment.
After irradiation, a regressed choroidal metastasis displays spicular hypertrophy of the retinal pigment epithelium.
Most patients who develop posterior choroidal metastasis can either be closely monitored for tumor growth, followed for response to chemotherapy, or treated with external beam irradiation. Radioactive plaque radiotherapy is rarely needed. If chemotherapy is not an option, prompt external beam irradiation (typically 30-40 Gy), may offer the best chance for preservation of vision.
In those rare cases where the metastasis spreads to the iris, patients can develop severe glaucoma and may lose their eye. Thankfully, anterior segment (iris) metastases are rare. Since most cancers do not spread to the iris, most patients with intraocular, choroidal and orbital metastasis respond well to treatment and keep their vision.
“Ezmai had a lazy eye and we spoke to a health visitor and then took her to the pre-school vision team but they told me to come back in six months. I kept taking her but they couldn’t find anything wrong. My instincts told me there was something wrong with Ezmai but I felt no-one believed me and that I was going mad.”
Beasley said she took Ezmai to four different health care providers, but the child was not diagnosed with retinoblastoma until she was two. By then, the cancer had advanced to the point that surgeons had to remove her eye in order to save her life.
Beasley has now teamed up with the The Childhood Eye Cancer Trust in Great Britain to raise awareness of the importance of early diagnosis of retinoblastoma. According to the organization, 21% of children diagnosed with this rare form of cancer in 2015 experienced a delay of at least six months between their first visit to a general practitioner and examination by a specialist.
“We looked back at an old photo of Ezmai at Christmas in 2014 and we can see a white glow in her eye but at the time we had absolutely no idea this could mean she had cancer,” Beasley said.
The Eye Cancer Foundation offers a free-downloadable retinoblastoma poster and a program, and sponsors a program to teach fellows to become retinoblastoma specialists in unserved and underserved countries. Dr. Paul Finger said it is imperative to make medical professionals more aware of the symptoms.
“Clearly, early detection and prompt treatment of children with retinoblastoma is crucial for saving both their vision and life.”
If you notice any of unusual symptoms in your children, you should have them examined by a specialist as soon as possible. Trust your instincts and don’t allow doctors who are not trained in detecting eye cancer dissuade you from getting an examination by a specialist.
This is definitely a situation where you would rather be safe than sorry.
"Very well treated by Dr. Finger. He explained everything I needed to know about my issue with detail and attention, putting me at ease and giving me confidence to handle this problem for the rest of my life.”